In a clinical study, Entrectinib (RXDX-101) treatment resulted in rapid response and tumor necrosis in a patient with small intestine neuroendocrine tumor who harbored a ETV6-NTRK3 fusion (PMID: 29118225).

In a preclinical study, transformed cells expressing the fusion, ETV6-NTRK3, were treated with Lestaurtinib (CEP-701), which resulted in inhibition of ETV6-NTRK3 autophosphorylation and restoration of Tgf-b1 signaling (PMID: 16258068).

In a preclinical study, transformed human cells expressing ETV6-NTRK3 were sensitive to BMS-536924, demonstrating a reduction in both cell survival and transformation activity in culture (PMID: 21804605).

In a clinical study, a patient-derived xenograft model with AML harboring ETV6-NTRK2 showed sensitivity to treatment with Larotrectinib, demonstrating a decrease in ETV6-NTRK2-expressing cells, and the patient with secondary AML, from whom the model was derived from, developed a partial remission when treated with Vitrakvi (larotrectinib), showing complete elimination of the cell population harboring ETV6-NTRK2 (PMID: 29920189).